Docs Give 2-year-old with Rare Liver Disease Reason to Smile

Paras smiles constantly as though to compensate for not doing so during the whole of last year. His huge abdomen had made it impossible for him to eat, sleep or smile. At two years and three months, this child from Belgaum had undergone a lifetime of medical misery–periodic draining of the abdomen liquid, trips to various doctors, and many homegrown medications.

Suffering from a rare liver disease called the Budd–Chiari Syndrome, Paras’s parents Dattaram and Shipa Madali not only waited for months before getting the right diagnosis, they were told that it was impossible to cure. They were advised that it was too risky to unclog his blocked liver (hepatic) veins, a manifestation of the syndrome. “There was a period in which we made peace with the inevitable, but during New Year’s Eve I couldn’t bear to see his plight,” said Dattaram. That is when, through their family doctor’s friends in Mumbai, they consulted Dr Aabha Nagral of Jaslok Hospital.

But Paras was a revelation for the medical team of Dr Nagral, paediatrician Dr Fazal Nabi and interventional radiologist Dr Shaji Marar–who last month published a paper in the Journal of Pediatric Gastroenterology and Nutrition on treating Budd–Chiari children. When the entire fluid from Paras’s abdomen was drained, the 11–kg child shrunk to 6 kg. “He is perhaps the lightest child to have undergone a surgical procedure for Budd–Chiari,” they say. None of the 16 children they have treated so far were as weak. It made his medical management in the ICU and OT that much more difficult.

Passing a catheter through a vein in Paras’s neck, Dr Marar created a direct connection of sorts between the portal vein and the inferior vena cava (the largest vein in the body) to allow blood to flow through Paras’s liver once again.

While getting discharged on Wednesday, the Madalis were happy with their smiling baby, but Dr Nagral is worried about the poor diagnosis of the syndrome among children. “The syndrome manifests in some children after a bout of fever and dehydration. For some reason, the blood becomes less viscous and clots in the hepatic veins, which have the slowest flow. Many of these children are wrongly diagnosed as liver failure patients and prescribed a transplant,” she says.

The poor awareness is apparent from the case of Jui, the six–year–old daughter of Dadar residents Madhura and Vidhyadhar Mhatre. “Jui’s abdomen started bloating when she was little over one,” recalls Madhura. The family went to several doctors and hospitals before going to KEM Hospital in Pune where they got the right diagnosis a year later. “She went through an angioplasty of one of the liver veins in a city hospital, but the liquid started filling up in her abdomen the very next day,” says her mother. They returned to the Pune doctor, who directed them to the Jaslok team. “Jui has a stent in one of her liver veins and has not had any problems in the last three years,” says Madhura.

Liver surgeon Dr Hemant Vadeyar from Kokilaben Dhirubhai Ambani Hospital says that Budd–Chiari among children is rare. “Not only is the incidence one in a lakh, there are few doctors qualified to carry out the needed intervention. While newer minimally invasive surgeries are available, they are expensive,” he says. Incidentally, Paras underwent a transjugular intrahepatic portosystemic shunt (TIPS). Dr Hemant Deshmukh, head of the interventional radiology department at KEM Hospital in Parel says that “doing TIPS in a child is a morbid procedure. The outcome is guarded.”

Understanding Budd–Chiari Syndrome

In 2 years, Paras has undergone a lifetime of medical misery

WHAT Budd–Chiari Syndrome is a rare problem that results from blood clotting in the veins flowing out of the liver (hepatic veins). The high pressure of blood in these veins leads to an enlarged liver, and to an accumulation of fluid in the abdomen, called ascites

SYMPTOMS Major symptoms include pain in the upper right–hand portion of the abdomen and a build–up of fluid in the abdomen

Six-year-old Jui has a stent in one of her liver veins

CAUSES Blood disorders are the most common causes, but in older people there could be various reasons from sickle cell anaemia, liver cancer\failure, infection, injury, etc

TREATMENT Patients undergo hepatic vein catheterisation in which a narrow tube is passed through the neck (via the right internal jugular vein) until it reaches the hepatic veins. An instrument at the tip of the catheter measures the pressure within each segment of the hepatic vein | In the same procedure, the surgeon can re–route blood flow around the clotted hepatic vein into a large vein called the vena cava| At times, doctors use a stent to remove the blockage. In worst cases, liver transplantation is performed | In a few patients, a ‘balloon catheter’ can open the blocked blood vessels without major surgery

INCIDENCE It is extremely rare. Congenital forms of Budd–Chiari syndrome are the most common cause of the syndrome worldwide, particularly in Asia. A study in Sweden reports an incidence of about 1 case per million population per year.It is rare in the general population and even more so in children. Peak incidence seems to be in persons aged 40–50 years

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